scholarly journals CLINICAL AND SEROLOGICAL PARAMETERS OF PROGRESSION AND PROGNOSIS IN PATIENTS WITH SYSTEMIC SCLEROSIS – A STATE OF THE ART REVIEW

2020 ◽  
Vol 73 (7) ◽  
pp. 1528-1532
Author(s):  
Ewa Wielosz ◽  
Maria Majdan
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Rna Polymerase ◽  
Connective Tissue Disease ◽  
State Of The Art ◽  
Rna Polymerase Iii ◽  
Vascular Complications ◽  
Skin Lesions ◽  
Tendon Ruptures ◽  
Generalized Type

Systemic sclerosis (SSc) is a multi-organ connective tissue disease that leads to the dysfunction and the impaired morphology of blood vessels due to non-specific inflammation and progressive fibrosis. Due to the diversity of SSc and even though the factors predisposing to the severe course of SSc are known, it is not always possible to predict the disease progression and to determine the prognosis. Ideally, the group of patients with faster progression of organ lesions and a worse course of the disease should be identified and the early intensive treatment should be instituted. The aim of the article, is an attempt to identify the factors that worsen the prognosis in the course of SSc. The analysis of numerous studies demonstrated that patients with short-lasting SSc, with the presence of anti-RNA polymerase III antibodies, with a generalized type of SSc with quickly progressing skin lesions and males should be most strictly monitored. Moreover, vascular complications, tendon ruptures and fast capillaries loss observed in nailfold capillaroscopy are the factors deteriorating the prognosis in SSc. In conclusion, despite the known, the factors that worsen the prognosis, it is difficult to predict the course of systemic sclerosis. Due to its incompletely elucidated etiopathology as well as the diverse and unpredictable nature of the disease, reliable markers to determine the prognosis in SSc have not been found.

scholarly journals Anti-Centromere Antibody Positivity in a Patient with Generalized Morphea

2018 ◽  
Vol 10 (3) ◽  
pp. 226-230
Author(s):  
Fumi Miyagawa ◽  
Anna Nakajima ◽  
Yasuhiro Akai ◽  
Hideo Asada
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Rna Polymerase Iii ◽  
Skin Lesions ◽  
Histological Findings ◽  
Skin Manifestations ◽  
Polymerase Iii ◽  
Oral Steroids ◽  
Antibody Positivity

We report the case of a 45-year-old female with generalized morphea (GM), who exhibited positivity for the anti-centromere antibody (Ab). She frequently developed multiple sclerotic skin lesions, whose histological findings were compatible with morphea. She demonstrated favorable responses to topical and oral steroids. Cases of GM associated with systemic sclerosis (SSc)-specific Abs (anti-Scl-70 Ab, anti-centromere Ab, and anti-RNA polymerase III Ab) have rarely been reported. The previously reported GM cases involving anti-SSc-specific Abs exhibited some skin manifestations of SSc, such as nailfold capillary changes. However, our case did not show any signs of SSc or limited cutaneous SSc. More cases are needed to clarify whether GM with SSc-specific Abs leads to SSc.

scholarly journals Relationship between type of skin lesions and nailfold capillaroscopy pattern in mixed connective tissue disease

2021 ◽  
Author(s):  
Anna Felis-Giemza ◽  
Sylwia Ornowska ◽  
Ewa Haładyj ◽  
Zenobia Czuszyńska ◽  
Marzena Olesińska
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Clinical Picture ◽  
Mixed Connective Tissue Disease ◽  
Skin Lesions ◽  
Systemic Lupus ◽  
Skin Changes

Abstract Introduction Mixed connective tissue disease (MCTD) is a rare disease with clinical picture consisted of multiple organ manifestations, including skin changes resembling systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or dermatomyositis (DM). On the background of these manifestations are microvascular changes — alteration of endothelial function and impairment of endothelial progenitor cell. Nailfold capillaroscopy (NFC) is a simple, non-invasive technique for investigating microvascular involvement in rheumatic diseases. Objectives To describe the relationship between type of skin lesions and NFC pattern in MCTD patients. Methods We analyzed the clinical picture and NFC patterns in 79 patients with MCTD. The NFC changes were classified into Normal, “Early,” “Active,” and “Late” scleroderma-like patterns (SD-like pattern) based on Cutolo classification. In all patients, subjective and physical examinations were carried out, specifically the occurrence of skin lesions in the course of MCTD was assessed (systemic sclerosis-like (Ssc-like), systemic lupus erythematosus-like (SLE-like), dermatomysitis-like (DM-like)). Results Skin changes were present in 64 (81%) patients, involving 43 (54%) SLE-like, 48 (61%) SSc-like, and 4 (5.1%) DM-like. NFC changes were observed in a total of 55 (69.6 %) patients with predominance of the “Early” pattern — 41 (51.9 %) patients. According to skin change phenotypes, NFC changes were observed in 31 (72%) patients with SLE-like and in 32 (66.7%) patients with SSc-like skin phenotypes. The “early” pattern predominated in both group. Conclusions We did not find any correlation between NFC pattern and the type skin changes. Key Points• The study did not show a correlation between the presence and absence of skin lesions and NFC pattern.• Scleroderma-like patterns were found in over 60% of patients with mixed connective tissue disease.• The “early” pattern is dominant regardless of the occurrence or absence of skin lesions in patients with MCTD.• Skin lesions, regardless of their type (SLE or SSc), do not correlate with type of lesion found in the NFC examination.

Association of anti-RNA polymerase III antibody and silicone breast implants in patients with systemic sclerosis

2016 ◽  
Vol 43 (7) ◽  
pp. 808-810 ◽  
Author(s):  
Ryosuke Saigusa ◽  
Yoshihide Asano ◽  
Kouki Nakamura ◽  
Takashi Yamashita ◽  
Yohei Ichimura ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Rna Polymerase Iii ◽  
Breast Implants ◽  
Silicone Breast Implants ◽  
Polymerase Iii

scholarly journals A longitudinal study of anti-RNA polymerase III antibody levels in systemic sclerosis

Rheumatology ◽  
2009 ◽  
Vol 48 (10) ◽  
pp. 1218-1221 ◽  
Author(s):  
S. I. Nihtyanova ◽  
J. C. Parker ◽  
C. M. Black ◽  
C. C. Bunn ◽  
C. P. Denton
Keyword(s):  
Longitudinal Study ◽  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Rna Polymerase Iii ◽  
Polymerase Iii ◽  
Antibody Levels

scholarly journals AB0668 ASSOCIATION OF ANTI-RNA POLYMERASE III ANTIBODY AND BREAST IMPLANTS RUPTURE IN ITALIAN PATIENTS WITH SYSTEMIC SCLEROSIS

2019 ◽  
Author(s):  
Maria Grazia Lazzaroni ◽  
Cristian Caimmi ◽  
Eugenia Bertoldo ◽  
Franco Franceschini ◽  
Angela Tincani ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Rna Polymerase Iii ◽  
Breast Implants ◽  
Polymerase Iii

Analysis of Anti-RNA Polymerase III Antibody-positive Systemic Sclerosis and Altered GPATCH2L and CTNND2 Expression in Scleroderma Renal Crisis

2020 ◽  
Vol 47 (11) ◽  
pp. 1668-1677
Author(s):  
Edward P. Stern ◽  
Sandra G. Guerra ◽  
Harry Chinque ◽  
Vanessa Acquaah ◽  
David González-Serna ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Renal Biopsy ◽  
Genetic Susceptibility ◽  
Rna Polymerase Iii ◽  
Human Kidney ◽  
Scleroderma Renal Crisis ◽  
Nucleotide Polymorphisms ◽  
Polymerase Iii ◽  
Renal Crisis

ObjectiveScleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) strongly associated with anti-RNA polymerase III antibody (ARA) autoantibodies. We investigated genetic susceptibility and altered protein expression in renal biopsy specimens in ARA-positive patients with SRC.MethodsARA-positive patients (n = 99) with at least 5 years’ follow-up (49% with a history of SRC) were selected from a well characterized SSc cohort (n = 2254). Cases were genotyped using the Illumina Human Omni-express chip. Based on initial regression analysis, 9 single-nucleotide polymorphisms (SNP) were chosen for validation in a separate cohort of 256 ARA-positive patients (40 with SRC). Immunostaining of tissue sections from SRC or control kidney was used to quantify expression of candidate proteins based upon genetic analysis of the discovery cohort.ResultsAnalysis of 641,489 SNP suggested association of POU2F1 (rs2093658; P = 1.98 × 10−5), CTNND2 (rs1859082; P = 5.58 × 10−5), HECW2 (rs16849716; P = 1.2 × 10−4), and GPATCH2L (rs935332; P = 4.92 × 10−5) with SRC. Further, the validation cohort showed an association between rs935332 within the GPATCH2L region, with SRC (P = 0.025). Immunostaining of renal biopsy sections showed increased tubular expression of GPATCH2L (P = 0.026) and glomerular expression of CTNND2 (P = 0.026) in SRC samples (n = 8) compared with normal human kidney controls (n = 8), despite absence of any genetic replication for the associated SNP.ConclusionIncreased expression of 2 candidate proteins, GPATCH2L and CTNND2, in SRC compared with control kidney suggests a potential role in pathogenesis of SRC. For GPATCH2L, this may reflect genetic susceptibility in ARA-positive patients with SSc based upon 2 independent cohorts.

scholarly journals Malignancies in Italian Patients with Systemic Sclerosis Positive for Anti-RNA Polymerase III Antibodies

2011 ◽  
Vol 38 (7) ◽  
pp. 1329-1334 ◽  
Author(s):  
PAOLO AIRO’ ◽  
ANGELA CERIBELLI ◽  
ILARIA CAVAZZANA ◽  
MARA TARABORELLI ◽  
STEFANIA ZINGARELLI ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Temporal Relationship ◽  
Rna Polymerase Iii ◽  
Patients With Cancer ◽  
Polymerase Iii ◽  
Number Of Patients ◽  
I 11 ◽  
Anticentromere Antibodies ◽  
Close Temporal Relationship

Objective.To evaluate the frequency of malignancies in Italian patients with systemic sclerosis (SSc) and anti-RNA polymerase III (RNAP III), antitopoisomerase I (topo I), or anticentromere antibodies (ACA); and to characterize the temporal relationship between the 2 diseases, in order to confirm data suggesting a close temporal relationship between the onset of SSc and malignancy in American patients with anti-RNAP III antibodies.Methods.From a cohort of 466 consecutive SSc patients, 360 Italians with isolated positivity for anti-RNAP III (n = 16), anti-topo I (n = 101), or ACA (n = 243) were identified. Malignancy cases were divided according to their relationship with SSc onset into 3 categories: preceding, synchronous with, or metachronous to the onset of SSc (diagnosed more than 6 months before; 6 months before to 12 months after; and more than 12 months after onset of SSc, respectively).Results.Malignancies were more frequent in the anti-RNAP III group (7/16 patients), than in the anti-topo I (11/101) and ACA groups (21/243) (p < 0.001). This difference was accounted for by the number of patients with cancer synchronous to the onset of SSc (3/16 in the anti-RNAP III group vs 0/101 in the anti-topo I and 1/243 in the ACA group; p < 0.001), whereas neither the number of malignancies preceding nor those metachronous to the onset of SSc was significantly different between the groups.Conclusion.In a cohort of Italian patients with SSc we observed a significant association between malignancies synchronous to SSc onset and positivity for anti-RNAP III antibodies, similar to that described in American patients with SSc.

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